Nutcracker syndrome combined with IgA Nephropathy: a cause of recurrent hematuria and proteinuria. Case report

The nutcracker syndrome is caused by the obstruction of the left renal vein secondary to its compression between the superior mesenteric artery and the aorta. Clinically, this syndrome manifests with pain, hematuria, varicocele or lower urinary tract symptoms. The prevalence of this syndrome is currently unknown; however, the diagnosis of this condition has increased thanks to the availability of non-invasive studies that allow its recognition. On the other hand, IgA nephropathy is the most common type of glomerular disease worldwide. Almost 15% of the causes of hematuria in children are secondary to this condition. The combination of IgA nephropathy and the nutcracker syndrome is rare. In the current literature, few cases have been described. We described the case of a 9-year-old scholar with hematuria and persistent proteinuria in the course of a purpura of Henoch-Schonlein in whom renal biopsy documented IgA nephropathy. However, during follow-up, due to the persistence of hematuria and proteinuria, in addition to other symptoms, additional studies are carried out confirming a Nutcracker syndrome. It is key in patients with persistent symptoms to rule out the association of these two diseases to avoid unnecessary interventions

Long-term follow-up of pediatric patients with nephrotic syndrome treated at Hospital Universitario San Vicente de Paúl (Medellín (HUSVP), Colombia), between January 1960 and December 2009

ABSTRACT: Nephrotic syndrome (NS) is a glomerular disease that frequently affects children. There have been few studies on it in Colombia. Objective: To describe the clinical and epidemiological features of children with SN treated at HUSVP between 1960 and 2009. Methodology: Retrospective and descriptive study. Results: Steroid-sensitive nephrotic syndrome was diagnosed in 87.9% of the patients, and between 1.7%-5.4% turned steroid-resistant. Biopsies revealed disease with minimal changes in 43.6% and focal segmental glomerulosclerosis in 37.3%. Additional immunosuppressive therapy was required by 40% of the patients; in 88.8% of these, cyclophosphamide was used, and remission was achieved in 85.7%. In 56% of the cases there were complications that were infectious in 52%. Nine percent of the patients progressed to end-stage renal disease. Mortality rate was 5.7%. Discussion: The large number of patients with nephrotic syndrome studied in this series and the long period of follow-up (up to 35 years) provide valuable information about the clinical behavior of this syndrome in Colombia and on its response to immunosuppressive therapy.RESUMEN: El síndrome nefrótico (SN) es una de las enfermedades glomerulares más frecuentes en la infancia y son pocos los estudios realizados en Colombia sobre esta enfermedad. Objetivo: describir las características clínicas y epidemiológicas de los niños con SN atendidos en el Hospital Universitario San Vicente de Paúl, de Medellín, Colombia, entre los años 1960-2009. Metodología: estudio descriptivo retrospectivo. Resultados: el 87,9% de los pacientes fueron corticosensibles, entre 1,7%-5,4% se tornaron corticorresistentes. La histopatología predominante fue la del síndrome nefrótico con cambios mínimos (43,6%) seguida por la glomeruloesclerosis focal y segmentaria (37,3%). El 40% requirieron inmunosupresión adicional; en 88,8% de estos se utilizó ciclofosfamida con remisión en 85,7%. Se presentaron complicaciones en 56% y 52% de ellas fueron infecciosas. El 9% de los pacientes llegaron a la insuficiencia renal crónica. La tasa de mortalidad fue del 5,7%. Discusión: esta es una de las series más grandes de pacientes con SN reportadas hasta el momento y con un período de seguimiento de hasta 35 años, lo que aporta información valiosa sobre el comportamiento local de la enfermedad y la respuesta al tratamiento inmunosupresor, El seguimiento a largo plazo de estos pacientes es fundamental para ofrecer el mejor tratamiento disponible y nos plantea nuevos interrogantes sobre la evolución del SN en nuestra población

Etiology of chronic kidney disease: fifty-year experience (1960-2010) at the Pediatric Nephrology Service, Hospital Universitario San Vicente de Paúl (Medellín, Colombia)

ABSTRACT: Chronic renal failure (CRF) results from progressive and irreversible deterioration of kidney function; it manifests as the inability to remove waste products and to maintain acid-base balance. The objectives of this study were to identify the diseases responsible for CRF in our patients and their relative importance in such outcome. A descriptive retrospective study was carried out, based on the review of the charts of 367 children with the diagnosis of CRF treated at the Pediatric Nephrology Service (Hospital Universitario San Vicente de Paúl, Medellín, Colombia) between 01 January 1960 and August 30, 2010. One hundred and ninety nine patients (54,2%) were males, and 168 (45,8), females. The predominant diseases as cause of CRF were: vesico-ureteral reflux (37.1%), hydronephrosis (24.0%), posterior urethral valves (13.4%), acute glomerulonephritis (12.3%), nephrotic syndrome (11.2%), and kidney hypoplasia (9.3%).RESUMEN: La insuficiencia renal crónica es el resultado del deterioro progresivo e irreversible de la función renal, que genera incapacidad del riñón para remover los productos de desecho y mantener el equilibrio ácido básico. El objetivo de este estudio fue identificar las causas de insuficiencia renal crónica en nuestros pacientes y su importancia relativa en este desenlace. Se hizo un estudio descriptivo retrospectivo de corte transversal, basado en la revisión de 367 registros pertenecientes al Servicio de Nefrología Infantil, de pacientes con diagnóstico de insuficiencia renal crónica (IRC), que acudieron al Servicio de Consulta Externa del Hospital Universitario San Vicente de Paúl, de Medellín, entre el 01 de enero de 1960 y el 30 de agosto de 2010. De los 367 pacientes, 199 (54,2%) fueron hombres y 168 (45,8%), mujeres; las enfermedades predominantes como causa de la IRC fueron: reflujo vésico-ureteral (37,1%), hidronefrosis (24,0%), valvas de la uretra posterior (13,4%), glomerulonefritis aguda (12,3%), síndrome nefrótico (11,2%) e hipoplasia renal (9,3%)

Use of rituximab in pediatric patients with steroid-resistant nephrotic syndrome. A single center study

ABSTRACT: Steroids are the cornerstone of therapy for nephrotic syndrome (NS) with a remission rate as high as 90%. In patients who do not respond to them or are steroid dependent, other immunosuppressive drugs have been used. Although rituximab use in NS is off-label, many authors have published their experience with it. Objective and methods: To describe retrospectively a group of seven children with nephrotic syndrome, either steroid-dependent (SDNS) or steroid-resistant (SRNS), treated with rituximab and mycophenolate, at Pablo Tobón Uribe Hospital, in Medellín, Colombia. Results: Two patients with SDNS and five with SRNS were evaluated; median age at diagnosis was 2 years (p25-75: 1-5); six months after treatment with rituximab there was reduction in proteinuria (93%), in the steroid dose (100%) and in the relapse episodes. However, proteinuria reappeared 12 months after treatment. Conclusion: During the first year after rituximab treatment of NS there is reduction in proteinuria and in the steroid dose, but thereafter there is relapse. It is suggested to carry out another study using a second dose of rituximab one year after the first one.RESUMEN: Los esteroides son el tratamiento de elección del síndrome nefrótico con tasas de éxito hasta del 90%. En pacientes que no responden adecuadamente a ellos, se han usado diferentes esquemas de inmunosupresión. Objetivo y métodos: describir la respuesta terapéutica en un grupo de siete niños con síndrome nefrótico córtico-dependiente (SNCD) o córtico-resistente (SNCR) que recibieron tratamiento con rituximab y micofenolato mofetil en un hospital universitario de la ciudad de Medellín durante los años 2010-2012. Resultados: dos pacientes tenían SNCD y cinco, SNCR; la mediana de edad en el momento del diagnóstico fue de 2 años (p25-75: 1-5); seis meses después de la aplicación del rituximab se encontró disminución de la proteinuria en el 93% de los pacientes; los esteroides se lograron suspender en el 100%; además, disminuyó el número de recaídas; sin embargo, la proteinuria reapareció un año después de dicho tratamiento. Conclusión: con el rituximab disminuyen la proteinuria y la dosis de esteroides, pero la enfermedad recurre 12 meses después de usarlo. Se sugiere hacer otro estudio evaluando el efecto de una segunda dosis de rituximab al año de la primera

Associated urological malformations and development of chronic kidney disease in pediatric patients with urinary tract infection at San Vicente de Paúl Hospital (Medellín, Colombia) between 1960 and 2010 = Malformaciones urológicas asociadas y desarrollo de enfermedad renal crónica en pacientes pediátricos con diagnóstico de infección urinaria que consultaron al Hospital Universitario San Vicente de Paúl (Medellín, Colombia) entre los años 1960-2010

Introduction: Urinary tract infection (UTI) is a major cause of bacterial disease in the pediatric population. Associated factors such as vesicoureteral reflux (VUR), posterior urethral valves, neurogenic bladder and other anatomical malformations increase the likelihood of developing renal scarring and dysplasia/hypoplasia, which at the same time increase in the long term the risk of hypertension (HT), proteinuria and chronic kidney disease (CKD). Objective: To describe the malformations associated with the frequency of UTI and development of CKD in pediatric patients who consulted San Vicente de Paúl Hospital, in Medellin, Colombia, between 1960 and 2010. Methods: A descriptive, retrospective study in which the clinical records of 4.476 patients with UTI were evaluated. Results: Patients with urinary tract anomalies corresponded to 78.3% of the total (predominance of women: 52.8%). Primary VUR was found in 29.9%; out of these, 5.1% progressed to CKD. Neurogenic bladder was diagnosed in 8.6%, of which 70.8% were secondary to myelomeningocele and 4.9% developed CKD. The posterior urethral valves were present in 3.5% of the total sample, of which 28.5% developed CKD. Conclusion: UTI in the pediatric population is a marker of urinary tract malformation and the prognosis is determined by the presence of VUR, obstructive anomalies and/or renal dysplasia favoring renal scarring, and increasing the risk of hypertension, proteinuria and CKD. An appropriate diagnostic approach would be the basis to implement management strategies to prevent deterioration of renal function